MCC occurs predominantly in the elderly light-skinned population. It is most commonly located on sun-exposed areas of the skin with approximately 50% of cases occurring on the head and neck, 40% on the extremities and 10% on the trunk and genitalia. The tumor typically presents as a non-tender subcutaneous mass, most often nodular but also with a plaque-like appearance, sometimes surrounded by satellite lesions. The color of MCCs varies from flesh-colored to red to lilaceous. The clinical features most commonly associated with primary MCC tumors have been summarized using the acronym ‘‘AEIOU’’: asymptomatic or non-tender; expanding rapidly; immune suppressed; older than 50 years; and ultraviolet-exposed fair skin (6).
Epidemiologic data suggests that there are approximately 2500 new MCC cases per year within the EU; approximately 1000 of these patients will die from their disease. The incidence of MCC is considerably increasing: The reported incidence has more than tripled over the past 20 years (2). This increase can partially be explained by the demographic development since MCC usually affects the elderly; the median age at diagnosis is 70 years, and there is a 5- to 10-fold increase in incidence after age 70 as compared with an age less than 60 years (7). Thus, it is likely that in an ageing European population the impact of this deadly cancer will continue to increase.